Double outlet right ventricle

Echocardiographic examination of a newborn infant showed a double outlet right ventricle with subpulmonary ventricular septal defect. The left atrium was divided by a membrane-like structure into a proximal chamber receiving pulmonary veins and a distal compartment containing left atrial appendage. To our knowledge, this is the first report of cor triatriatum sinistrum associated with double outlet right venticle.

Double outlet right ventricle occurs in 0.4 to 1.0 percent of all congenital heart disease.[1] Cor tritriatum sinistrum is also rare.[2] The association of both anomalies is therefore expected to be very rare. We describe herein a newborn infant with double outlet right ventricle and cor triatriatum sinistrum.

CASE REPORT

A male infant presented with respiratory distress and a heart murmur of 15 h of age. Echocardiographic examination showed normal situs of the atria, concordant atrioventricular connection, double outlet right ventricle with aortic valve to the right, and side-by-side to pulmonary valve. There was large subpulmonary ventricular septal defect with the extension to the inlet septum (Fig 1). Infundibular septum deviated slightly toward the aorta but no subaortic gradient was detected with pulsed Doppler interrogation. The aortic valve was bicuspid but not stenotic. Tight preductal coarctation of the aorta with hypoplastic isthmus and gradient across coarctation of 50 mm Hg was detected. Ductus arteriosus was widely open. Both septal and anterosuperior leaflets of the tricuspid valve were proximally attached to malaligned infundibular septum, and the septal leaflet was anchored to the crest of the trabecular septum distally. In the left atrium, there was a membrane-like structure dividing it into two compartments. The upper part received pulmonary veins and was connected to the distal part by large nonobstructive opening (Fig 2.).

Cardiac catheterization and angiography confirmed the diagnosis and delineated the aortic arch more precisely. It showed, in addition to preductal coarctation, isthmal narrowing and aberrant right subclavian artery originating below the site of the coarctation. The levophase of right venticular injection failed to show the membrane in the left atrium. Prostaglandin infusion was started, and at the age of 3 days, coarctation of the aorta was repaired with patch and pulmonary artery was banded.

DISCUSSION

In patients with double outlet right ventricle, echocardiography can delineate in detail the origin of both great arteries from the right ventricle, their relationship to each other, size and position of the ventricular septal defect, presence or absence of mitral valve-semilunar valve continuity, anomalous atrioventricular valve attachment, morphologic features of infundibular septum, and additional cardiovascular abnormalities.[3] Not infrequently, lesions of the left ventricular inlet can be encountered. In pathologic study, they were found in 10 of 33 cases. Seven of them were obstructive. They consisted of supraventricular mitral ring, parachute mitral valve, mitral valvular atresia, straddling mitral valve, and adherence of the mitral valve leaflet to the edge of the ventricular septal defect.[4] Cor triatriatum sinistrum can also cause obstruction to the left ventricular inflow. It is usually an isolated lesion, but it can occur in association with other cardiovascular anomalies.[5]

To our knowledge, this anomaly has not yet been reported in combination with double outlet right ventricle. The diagnosis can be missed easily if only cardiac catheterization and angiography are done, especially if it is nonobstructive or associated with some other lesions.[6] It is also frequently impossible to differentiate it from other anomalies of the left ventricular inlet. Cor triatriatum may go unrecognized even with echocardiographic examination if associated with other cardiac defects that can mislead the echocardiographer who might concentrate only on these major defects. However, with the strict use of all the planes of cross-sectional examination, including Doppler and color Doppler, exact preoperative diagnosis could be made and unnecessary postoperative morbidity avoided, especially in cases where cor triatriatum causes the obstruction to the left ventricular inlet.

REFERENCES

[1] Hoffman JIE. Incidence, mortality and history. In: Anderson RH, Macartney FY, Shinebourne AE, Tynan M, eds. Paediatric cardiology. Edinburgh: Churchill Livingstone, 1987; 5

[2] Keith JD, Rowe RD, Vlad P. Heart disease in infancy and childhood. New York: Macmillan Publishing Co, 1978; 6

[3] Roberson DA, Silverman NH. Malaligned outlet septum with sub-pulmonary ventricular septal defect and abnormal ventriculoarterial connection: a morphologic spectrum defined echocardiographically. J Am Coll Cardiol 1990; 16:459-68

[4] Zamora R, Moller JH, Edwards JE. Double outlet right ventricle: anatomic types and associated anomalies. Chest 1975; 68:675-77

[5] Ostman-Smith I, Silverman NH, Oldershaw P, Lincoln C, Shinebourne EA. Cor triatriatum sinistrum: diagnostic features on cross sectional echocardiography. Br Heart J 1984; 51:211-19

[6] Jacobstein MD, Hierschfeld SS. Concealed left atrial membrane: pitfalls in the diagnosis of cor triatriatum and supravalve mitral ring. Am J Cardiol 1982; 49:780-86

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